Amplifiers co-translationally enhance CFTR biosynthesis via PCBP1-mediated regulation of CFTR mRNA

Cystic fibrosis (CF) is a life-shortening recessive disorder caused by mutations in both alleles of the cystic fibrosis transmembrane conductance regulator (CFTR) gene [1]. CFTR codes for an ion channel that mediates chloride transport across epithelial cell membranes. Mutations resulting in CFTR dysfunction cause chronic obstructive lung disease, intestinal obstruction syndromes, liver dysfunction, exocrine and endocrine pancreatic dysfunction, and male infertility [1,2]. CFTR is a complex, multidomain, membrane-spanning protein that undergoes highly regulated folding and trafficking post-biosynthesis to be functionally mature within epithelial cells [3].

Source: Journal of Cystic Fibrosis - February 15, 2020 Category: Respiratory Medicine Authors: Danijela Dukovski, Adriana Villella, Cecilia Bastos, Randall King, Daniel Finley, Jeffery W. Kelly, Richard I. Morimoto, F. Ulrich Hartl, Benito Munoz, Po-Shun Lee, Marija Zecevic, John Preston Miller Tags: Original Article Source Type: research